Trends in Incidence and Mortality of Waldenström Macroglobulinemia: A Population-Based Study

Abstract

Background: The incidence of Waldenström macroglobulinemia (WM) has increased in certain groups over several decades in the United States. It is unclear whether the increasing incidence is associated with mortality trends. Methods: The incidence and incidence-based mortality (IBM) rates were obtained from the Surveillance, Epidemiology, and End Results (SEER) database (1980-2016) with SEER * Stat software. The secular trends stratified by demographic characteristics were analyzed by joinpoint regression. Results: The incidence of WM showed an initial rapid increase from 1980 to 1993 {annual percentage change (APC), 14.1% [95% confidence interval (CI), 10 to 18.4%]}, whereas it began to stabilize from 1993 to 2016 [APC, 0.5% (95% CI, −0.3 to 1.3%)]. The WM IBM trend followed a similar pattern, with a decrease occurring around 1994. The trends in the incidence and mortality significantly differed according to geographic location, race, age, sex, primary site of involvement and subtype, which could help in further investigations into the specific etiology. Moreover, a dramatic increase in the 5-year survival rate from the 1980s to 2010s was observed (47.84 vs. 69.41%). Conclusions: Although both the incidence and IBM of WM continued to increase during the study period, a reduction in the rate of increase occurred around 1993. We believe that further advances in healthcare delivery and research can ensure a low mortality rate. Future studies can use the findings of this paper to monitor the results of WM therapy.

Primary therapy and relative survival in patients with lymphoplasmacytic lymphoma/Waldenström macroglobulinaemia: a population‐based study in the Netherlands, 1989–2018

“…3,21,22 In two other populationbased studies, the incidence initially increases until the late 1990s/early 2000s, after which it stabilised. 6,23 Unlike these studies, an increase in incidence was not observed in our present study. This difference could be due to stable registration and diagnostic practices in the Netherlands.…”

“…Both entities are hereafter collectively referred to as LPL/WM, a disease primarily affecting individuals aged >60 years 3 . The annual age‐standardised incidence rate (ASR) of LPL/WM is approximately three per million person‐years in Western countries 4–6 . The natural history of LPL/WM is heterogeneous, with many patients remaining asymptomatic for years without need for treatment, while others require treatment early 7 .…”

Treatment paradigm in Waldenström macroglobulinemia: frontline therapy and beyond

“…Surveillance, epidemiology, and end results data studies have demonstrated the annual incidence rate to be higher in Caucasian population (0.52 per 100,000; 95% CI: 0.50–0.53) compared with African American population (0.29 per 100,000, 95% CI: 0.26–0.33). 4 Waldenström macroglobulinemia lies on a spectrum of disorders ranging from an immunoglobulin M (IgM) monoclonal gammopathy of undetermined significance (MGUS), to asymptomatic/smoldering WM and eventually symptomatic disease. 1 The common underlying feature in these disorders is the presence of circulating monoclonal IgM protein.…”

The Use of Bruton Tyrosine Kinase Inhibitors in Waldenström’s Macroglobulinemia

“…It is a relatively uncommon non-Hodgkin lymphoma (NHL) with an incidence rate of 0.3 per 100,000 persons [ 2 ]. WM is a disease of the elderly, with a predilection for males more than females, and for Caucasians more than other populations [ 3 ]. There may be genetic and environmental factors which contribute to the development of WM, but no clear causative factors have been identified in the pathogenesis [ 4 ].…”

“…There may be genetic and environmental factors which contribute to the development of WM, but no clear causative factors have been identified in the pathogenesis [ 4 ]. Based on a recent Surveillance, Epidemiology, and End Results (SEER) analysis, the five-year survival rate has improved from 48% to 69% during the period from the 1980s to the 2010s [ 3 ].…”